整合世界卫生组织（WHO）2001-2008 年用于诊断骨髓增生异常综合征（MDS）的标准：苯暴露的病例对照分析。
Integrating WHO 2001-2008 criteria for the diagnosis of Myelodysplastic Syndrome (MDS): a case-case analysis of benzene exposure.
机构信息
Fudan-Cinpathogen Clinical and Molecular Research Center, Institutes of Biomedical Sciences, Fudan University, Shanghai, China.
出版信息
Chem Biol Interact. 2010 Mar 19;184(1-2):30-8. doi: 10.1016/j.cbi.2009.11.016. Epub 2009 Nov 24.
We characterized the prevalence of hematopoietic and lymphoid disease for 2923 consecutive patients presenting at 29 hospitals from August 2003 to June 2007. Diagnoses were made in our laboratory using WHO criteria based on morphologic, immunophenotypic, cytogenetic, FISH and molecular data. A total of 611 subjects (322 males/289 females) were prospectively diagnosed with MDS using WHO (2001) criteria. Update and re-evaluation of cases using MDS (2008) criteria resulted in 649 MDS cases. Using WHO (2008) criteria, refractory cytopenia with multilineage dysplasia (RCMD) accounted for 68% of total cases, refractory anemia with excess blasts (RAEB), 16.3%; refractory anemia (RA), 6.5%; refractory cytopenia with unilineage dysplasia (RCUD), 4%; and MDS-unclassifiable (MDS-U), 4.5%. Subjects were administered questionnaires and information on previous disease, work histories and exposures to potential etiologic agents such as benzene (BZ) was obtained. A total of 80/649 (13.2%) were determined to have some BZ exposure. The frequency of clonal cytogenetic abnormalities in all MDS was 30%, the most common being +8>del(20)q>del(7q)>del(5q), while the analogous frequency in BZ-exposed cases was only 24%. To further investigate the characteristics of MDS associated with BZ, we identified a subset of cases with high BZ exposure. These BZ signal cases were each matched by age and gender to two cases with no known BZ exposure. When contrasting BZ signal cases vs matched cases with no BZ exposure, we found a high odds ratio (OR) for the WHO subtype MDS-U (OR=11.1), followed by RAEB and RCUD (OR=1), RA (OR=0.7) and RCMD (OR=0.6). Multilineage dysplasia with abnormal eosinophils (MDS-Eo) was strongly associated with BZ exposure, whereas the relative risk of clonal cytogenetic abnormalities was reduced for high BZ-exposed cases (OR=0.5). These findings are strongly indicative that MDS subtypes are influenced by BZ exposure, and taken together with previous studies, the features of MDS-Eo suggest that altered immune regulation plays a major role in the pathogenesis of MDS following chronic exposure to BZ.
我们对 2003 年 8 月至 2007 年 6 月期间在 29 家医院就诊的 2923 例连续患者进行了造血和淋巴系统疾病的患病率特征分析。我们使用基于形态学、免疫表型、细胞遗传学、FISH 和分子数据的世界卫生组织（WHO）标准，在我们的实验室中做出诊断。共有 611 名患者（男性 322 名/女性 289 名）采用 WHO（2001）标准前瞻性诊断为 MDS。使用 MDS（2008）标准更新和重新评估病例后，共诊断出 649 例 MDS 病例。采用 WHO（2008）标准，难治性血细胞减少伴多系发育异常（RCMD）占总病例的 68%，难治性贫血伴原始细胞过多（RAEB）占 16.3%，难治性贫血（RA）占 6.5%，难治性血细胞减少伴单系发育异常（RCUD）占 4%，MDS 无法分类（MDS-U）占 4.5%。我们向患者发放了调查问卷，并收集了有关既往疾病、工作史以及接触苯（BZ）等潜在病因物质的信息。共有 80/649（13.2%）例被确定存在一定程度的 BZ 暴露。所有 MDS 中克隆细胞遗传学异常的频率为 30%，最常见的是+8>del（20）q>del（7q）>del（5q），而在 BZ 暴露病例中的类似频率仅为 24%。为了进一步研究与 BZ 相关的 MDS 的特征，我们确定了一组高 BZ 暴露病例。将这些 BZ 信号病例分别与年龄和性别匹配的两名无已知 BZ 暴露病例进行对比。当对比 BZ 信号病例与无 BZ 暴露的匹配病例时，我们发现 WHO 亚型 MDS-U（OR=11.1）的比值比（OR）较高，其次是 RAEB 和 RCUD（OR=1），RA（OR=0.7）和 RCMD（OR=0.6）。伴有异常嗜酸性粒细胞的多系发育异常（MDS-Eo）与 BZ 暴露密切相关，而高 BZ 暴露病例的克隆细胞遗传学异常的相对风险降低（OR=0.5）。这些发现强烈表明 MDS 亚型受 BZ 暴露的影响，结合以往的研究，MDS-Eo 的特征表明，慢性接触 BZ 后，免疫调节的改变可能在 MDS 的发病机制中起主要作用。
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